Hemidiaphragmatic paralysis increases postoperative morbidity after a modified Fontan operation.

OBJECTIVES: After a Fontan procedure, forward pulmonary blood flow is augmented during inspiration because of negative intrathoracic pressure. Total pulmonary blood flow is higher during inspiration. With hemidiaphragmatic paralysis, inspiratory augmentation of pulmonary flow is lost or diminished. The objective of this study was to compare early postoperative morbidity after the modified Fontan operation in patients with and without hemidiaphragmatic paralysis. METHODS: A case-control analysis was performed comparing 10 patients with documented hemidiaphragmatic paralysis against 30 patients without paralysis who were matched for diagnosis, fenestration, and age. The following early postoperative outcomes were assessed: duration of ventilator support, duration of hospital stay, incidence of ascites, prolonged effusions, and readmission. RESULTS: Preoperatively, there were no significant differences between the 2 groups. However, among the postoperative outcomes, the duration of hospital stay (25.4 +/- 16.6 days vs 10.8 +/- 6.3 days; P =.03), incidence of ascites (70% vs 3%; P <.001), prolonged pleural effusions (60% vs 13%; P =.007), and readmission (50% vs 7%; P =.007) were significantly greater in patients with hemidiaphragmatic paralysis than in those without hemidiaphragmatic paralysis. CONCLUSIONS: Hemidiaphragmatic paralysis after the modified Fontan operation is associated with an increase in early morbidity. Care should be taken to avoid injury to the phrenic nerve. Patients with prolonged effusions should be evaluated for hemidiaphragmatic paralysis.

Pre-operative coagulopathy management of a neonate with complex congenital heart disease: a case study.

Severe coagulation defects often develop in neonates undergoing cardiac surgery, both as a result of the surgical intervention, and as pre-existing defects in the hemostatic mechanisms. The following case report describes a newborn patient with complex congenital heart disease and respiratory failure whose pre-operative coagulopathy was aggressively managed prior to surgical correction. A 5-day-old, 2.5 kg child presented with interrupted aortic arch, ventricular septal defect, atrial septal defect, and patent ductus arteriosus. On admission, he was in respiratory arrest suffering from profound acidemia. In addition, the child was hypothermic (30.1 degrees C), septic (Streptococcus viridans), and coagulopathic (disseminated intravascular coagulation-DIC). The patient was immediately intubated and initial coagulation assessment revealed the following: prothrombin time (PT) 48.9 s (international normalized ratio (INR) 15.7), activated partial thromboplastin time (aPTT) >106 s, platelet count 30,000 mm(3), fibrinogen 15 mg dL(-1) and antithrombin III (AT-III) 10%. Before cardiac surgery could be performed, the patient's DIC was corrected with the administration of cryoprecipitate (15 ml), fresh frozen plasma (300 ml), and platelets (195 ml). In spite of the large transfusion of fresh frozen plasma, the AT-III activity, measured as a percentage, remained depressed at 33. Initial thromboelastographic (TEG) determination revealed an index of +2.02, and following 100 IU administration of an AT-III concentrate, declined to -2.32. Sequential TEG profiles were performed over several days, with the results used to guide both transfusion and medical therapy. The congenital heart defect correction was subsequently performed with satisfactory initial results, but the patient developed a fungal infection and expired on the 16th post-operative day. The present case describes techniques of coagulation management for a newborn with both a severe hemostatic defect and congenital heart disease.

Results with the Mustard operation in simple transposition of the great arteries 1963-1985.

From May 1963 to December 1985, 329 infants and children underwent repair of simple transposition of the great arteries (TGA) using Mustard's technique. To assess improvement, the patients were separated into two groups by date of operation: Group I, May 1963 to December 1973 (N = 106), and Group II, January 1974 to December 1985 (N = 223). The operative mortality was 11 (10.4%) in Group I and two (0.9%) in Group II. The 10-year actuarial survival rate was 73.4% in Group I and 93.7% in Group II. Baffle complications, similar in both groups, were identified in 81 patients; 19 were major, causing death or requiring reoperation. By latest electrocardiogram, 21 of 45 (46.7%) of Group I patients and 129 of 180 (71.7%) of Group II patients were in normal sinus rhythm. Late ambulatory electrocardiography, however, revealed that a majority of patients had sinus node dysfunction or other dysrhythmias. Right ventricular (RV) angiography revealed definite diminution of RV contractility in 14 (11%) of 126 children. At late follow-up, 113 of 148 (76%) children were in New York Heart Association (NYHA) Class I and 35 of 148 (24%) were in NYHA Class II. Thirty-five (21%) patients were on cardiac medication chiefly for dysrhythmia management. There has been significant improvement in the early and late mortality with the Mustard operation, and serious baffle complications are infrequent. Dysrhythmias continue to be a major problem but the surviving patients are clinically well and relatively few have significant RV dysfunction.

Surgical experience with reparative techniques in patients with congenital mitral valvular anomalies.

Our review of an entire institutional experience with primary repair of congenital and acquired mitral valve (MV) anomalies in children with concordant atrial-ventricular-arterial connections but without atrioventricular septal defects included 48 patients (1962 to September 1986). Mitral stenosis (MS) was the predominate lesion in 24 patients, mitral incompetence in 22, and mixed in two. Most patients with MS had so-called typical congenital MS (Van Praagh) with abnormalities of all valvular components; virtual or complete absence of chordal development was a consistent finding. Other anatomic substrates (not mutually exclusive) producing MS included supramitral ring with Shone's complex (n = 5), parachute mitral valve (n = 2), hypoplastic mitral ring (n = 3), and rheumatic lesions (n = 3). The anatomic substrates producing mitral incompetence included annular dilatation (n = 16; isolated in seven), leaflet prolapse (n = 7), cleft leaflet (n = 6), leaflet deficiency (n = 3), and rheumatic lesions (n = 2). Concurrent repair of associated lesions was performed in 29 (60%) patients for relief of left ventricular outflow tract obstruction (n = 16) and closure of ventricular septal defects (n = 9; double-outlet right ventricle in three). Six (12.5%) patients had previous repair of coarctation of the aorta. The operative mortality rate was 18.7% (70% confidence limits [CL]; 12.8% to 24.6%), with one death in 35 patients since 1975 (2.9%; 70% CL; 0% to 5.8%). The actuarial freedom from early or late death and reoperation (+/- SEM) was 44.0% +/- 14.8% at 8 years postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)